Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 2  |  Page : 185-186

Juxtarenal aortic occlusion with situs inversus totalis: Surgical management of a rare association


Department of Cardiovascular and Thoracic Surgery, Division of Vascular Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Date of Submission11-Dec-2019
Date of Decision12-Dec-2019
Date of Acceptance25-Dec-2019
Date of Web Publication17-Jun-2020

Correspondence Address:
Dr. Shivanesan Pitchai
Department of Cardiovascular and Thoracic Surgery, Division of Vascular Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_103_19

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  Abstract 


Situs inversus totalis (SIT) is a rare entity characterized by mirror-image reversal of the viscera. The coexistence of SIT and Leriche syndrome has been rarely described. The authors describe the surgical management of juxtarenal aortic occlusion in a patient with SIT which was successfully managed with open aortic endarterectomy and distal bypass. The malposed organs are at a greater risk of operative injury, but surgical management is feasible with proper determination of the anomalies on preoperative imaging.

Keywords: Aortic endarterectomy, juxtarenal aortic occlusion, left-sided inferior vena cava, Leriche syndrome, situs inversus totalis


How to cite this article:
Pandey AK, Nair HR, Kumar P M, Pitchai S. Juxtarenal aortic occlusion with situs inversus totalis: Surgical management of a rare association. Indian J Vasc Endovasc Surg 2020;7:185-6

How to cite this URL:
Pandey AK, Nair HR, Kumar P M, Pitchai S. Juxtarenal aortic occlusion with situs inversus totalis: Surgical management of a rare association. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2020 Jul 6];7:185-6. Available from: http://www.indjvascsurg.org/text.asp?2020/7/2/185/286901




  Introduction Top


Situs inversus characterized by transposition of viscera in thoracic and abdominal cavity is a seldom reported anomaly. The term “situs inversus totalis” (SIT) is used when dextrocardia is present along with visceral inversion.[1] The presence of a left-sided inferior vena cava (IVC) and difficulty posed by the altered bowel orientation renders aortic surgery challenging.[2] We report the successful management of juxtarenal aortic occlusion with aortobifemoral bypass in a case of SIT.


  Case Report Top


The index case is a 49 year old gentleman who presented with complaints of disabling claudication in the bilateral lower limbs along with erectile dysfunction for the past 6 months. He was recently started on insulin and oral hypoglycemics for poorly controlled Type 2 diabetes mellitus, and was also on medical management for hypertension with a single drug for the past 6 months. He had a 10 pack-year smoking history. Examination revealed absence of bilateral femoral pulses and noncompressible pedals, with bilateral ankle brachial index >1.2. Blood workup indicated increased glycosylated hemoglobin and dyslipidemia. Computed tomography imaging revealed long segment juxtarenal aortoiliac occlusion along with SIT [Figure 1]. On echocardiogram study along with dextrocardia, good left ventricular function and ejection fraction of 60% were noticed. He was optimized for aorto bifemoral bypass.
Figure 1: Preoperative computed tomography angiogram; (a) In the coronal image, the level of occlusion is seen just below the lower left renal arteries, inferior vena cava seen on the left, transposed liver and spleen seen; (b) Dextrocardia and descending aorta seen on the right side

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Standard midline approach was used for bypass; complete visceral transposition was noted [Figure 2]a. The transverse colon was retracted cephalad, and the small bowel loops were wrapped in a wet sheet and retracted to the left side as opposed to the conventional approach. IVC was encountered on the left side of the aorta. Suprarenal clamp space was created, and bilateral renal arteries were looped. Retro-ureteric tunneling was done for graft with a graft tunneler to the left groin and right external iliac artery. After heparinization, the clamp was applied above the left main renal artery and below the right renal artery, aortotomy was done, and the aorta was cleared of thrombus and atheromatous material. The clamp was shifted to the infrarenal aorta after declamping renal arteries for back bleed, followed by end-to-side anastomosis of the proximal limb of 16 mm × 8 mm polyester graft [Figure 2]b. The right distal end was tunneled to the external iliac artery and anastomosed in an end-to-side fashion and the left was anastomosed in a similar fashion to the left common femoral artery. The patient was monitored in the intensive care unit after surgery for a day and was discharged after an uneventful postoperative course on day 7. At 6-month follow-up, the bilateral lower limbs had palpable pedals, and he was symptomatically relieved.
Figure 2: Intraoperative images; (a) Supracoloic compartment; stomach seen on the right, gallbladder can be seen in the extreme left; (b) Completion of proximal anastomosis, inferior vena cava seen on the left side

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  Discussion Top


The first description of situs inversus in animals was by Aristotle, who described it as visitation from Gods. Fabricius in 1600 reported the first human case in a cadaver specimen and the term “situs inversus viscerum” was coined by Marco Severino in 1643.[1] SIT has been hypothesized to be the result of abnormalities in gastrulation at 3rd week which corresponds to the time of determination of embryonal axes.[2]

The clinical triad of aortoilliac occlusion entailing claudication, erectile dysfunction, and absent peripheral pulses was named after Leriche, who also proposed the management strategy for the same, who also proposed a management strategy for the same.[3] Infrarenal aortic occlusion may be classified as distal which typically spares inferior mesenteric artery origin, and proximal or juxtarenal, that extends cranially approaching the renal arteries.[4] Juxtarenal aortic endarterectomy with distal revascularization by bypass graft, first described by Bergan and Trippel in 1963, remains the gold standard for proximal aortic occlusions.[5] Surgical repair of juxtarenal occlusion though challenging provides unmatched long-term results and is regarded as the treatment of choice in surgically fit patients.[4]

The occurrence of Leriche syndrome along with SIT has been rarely reported.[6],[7] Literature review revealed a single case report of successful bypass in infarenal occlusion in situ s inversus.[6] We for the first time report the management of juxtarenal occlusion in a case of SIT.

The challenges posed by unfamiliar anatomy and transposed major veins in SIT can be overcome by a meticulous preoperative planning and analysis of the relevant anatomy, thereby ensuring a successful outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Severino MA. De recondita abscessuum natura: Libri VIII; 1643. p. 468.  Back to cited text no. 1
    
2.
Riera Hernández C, Pérez Ramírez P, Esteban Gracia C, Jiménez Olivera MA, Llagostera Pujol S. Situs inversus totalis and abdominal aortic aneurysm: Surgical repair of an extremely uncommon association. Int J Surg Case Rep 2015;10:216-8.  Back to cited text no. 2
    
3.
Leriche R, Morel A. The syndrome of thrombotic obliteration of the aortic bifurcation. Ann Surg 1948;127:193-206.  Back to cited text no. 3
    
4.
Marrocco-Trischitta MM, Bertoglio L, Tshomba Y, Kahlberg A, Marone EM, Chiesa R. The best treatment of juxtarenal aortic occlusion is and will be open surgery. J Cardiovasc Surg (Torino) 2012;53:307-12.  Back to cited text no. 4
    
5.
Bergan JJ, Trippel OH. Management of juxtarenal aortic occlusions. Arch Surg 1963;87:230-8.  Back to cited text no. 5
    
6.
Oliveira N, Oliveira F, Dias E, Borges L, Cássio I. Leriche syndrome in a patient with situs inversus totalis. Ann Vasc Surg 2015;29:1317.e5-8.  Back to cited text no. 6
    
7.
Rapovka VG. Occlusion of the infrarenal segment of the aorta in a patient with total situs inversus of the internal organs. Khirurgiia 1989;(3):137.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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